Hyper-IgE syndrome (HIES) is a rare primary immunodeficiency (<1 case per million population) characterized by elevated serum IgE levels, recurring staphylococcal skin abscesses, and pneumonias.1 Patients with HIES may show peripheral eosinophilia, eosinophilic infiltration of the skin, and increased susceptibility to infection.2 In newborns, it first manifests as an eruption3 that usually persists as a moderate to severe eczematoid dermatitis into childhood and adulthood. This multisystem disorder is associated with skeletal, facial, and dental abnormalities.1 We report the first case to our knowledge of a patient with HIES with severe recalcitrant eczematous dermatitis successfully treated with high-dose monoclonal anti-IgE therapy.
Clinical appearance of our patient before (A) and 6 months after stopping (B) monotherapy with omalizumab.
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